Familial Uncombable Hair Syndrome: Ultrastructural Hair Study and Response to Biotin

The study reported on a family affected by uncombable hair syndrome across four generations, characterized by unruly, dry, blond hair with a tangled appearance. The condition appeared to follow an autosomal dominant inheritance pattern, with some family members also showing minor signs of atopy and ectodermal dysplasia, such as nail abnormalities. Diagnosis was confirmed using scanning electron microscopy. A trial of oral biotin at 5 mg/day was conducted on two young patients, resulting in excellent improvements in hair appearance, although no structural changes were observed in the hair under microscopy. After 2 years of follow-up, the improved hair condition was maintained without biotin, but nail fragility still required biotin supplementation.